title: Cochlear implantation in 13q deletion syndrome.
abstract: OBJECTIVE
  The advent of cochlear implantation has been followed by an explosion in surgical and technological advances with subsequent alterations in the treatment of sensorineural hearing loss management. Many individuals with rare genetic abnormalities who once may have not been considered candidates for cochlear implantation are now benefiting from cochlear implantation. One of these unusual syndromes is the 13q deletion syndrome. The clinical features of 13q deletion syndrome, a rare chromosomal abnormality, were originally described in the early 1960s, though the literature currently lacks defined phenotypic abnormalities. Patients with 13q deletion syndrome present with varying degrees of cognitive and growth delay, craniofacial dysmorphisms, and congenital malformations. The etiology of the sensorineural hearing loss has not been elucidated, and genes associated with other syndromes which include sensorineural hearing loss have been proposed as the causative agents. We describe the unique clinical and surgical considerations for 13q deletion syndrome and review the considerations when deliberating on cochlear implantation in syndromic hearing loss.
  
  
  METHODS
  Case report.
  
  
  RESULTS
  Successful cochlear implantation in a patient with 13q deletion syndrome.
  
  
  CONCLUSIONS
  Many patients with 13q deletion syndrome suffer from sensorineural hearing loss, and when appropriately selected may likely benefit from cochlear implantation. Many other syndromic patients following careful selection may also benefit from cochlear implantation and should not be excluded from consideration until appropriately evaluated.
authors.affiliation: Georgia Regents University, Augusta, Georgia.
authors.forename: Angela
authors.initials: A
authors.lastname: Haskins
authors.forename: Alex
authors.initials: A
authors.lastname: Caten
authors.forename: Brian J
authors.initials: BJ
authors.lastname: McKinnon
mesh_terms: D002675:Child, Preschool; D002872:Chromosome Deletion; D025063:Chromosome Disorders; D002882:Chromosomes, Human, Pair 13; D019929:Cochlear Implantation; D006319:Hearing Loss, Sensorineural / Q000235:genetics* / Q000601:surgery*; D006801:Humans; D008297:Male